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Cognitive and behavioral profile of progressive supranuclear palsy and its phenotypes

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Abstract

Background

Although several progressive supranuclear palsy (PSP) phenotypes have recently been described, studies identifying cognitive and neuropsychiatric differences between them are lacking.

Methods

An extensive battery of cognitive and behavioural assessments was administered to 63 PSP patients, 25 PD patients with similar sociodemographic characteristics, and 25 healthy controls. We analysed differences in phenomenology, frequency and severity of cognitive and neuropsychiatric symptoms between PSP, PD and HC, and between PSP subtypes.

Results

Regarding phenotypes, 64.6% met criteria for Richardson’s syndrome (PSP-RS), 10.7% PSP with predominant Parkinsonism (PSP-P), 10.7% with PSP progressive gait freezing (PSP-PGF), and 10.7% PSP with predominant speech/language disorder (PSP-SL). Impairment was more severe in the PSP group than in the PD and HC groups regarding motor scores, cognitive testing and neuropsychiatric scales. Cognitive testing did not clearly differentiate between PSP phenotypes, but PSP-RS and PSP-SL appeared to have more cognitive impairment than PSP-PGF and PSP-P, mainly due to an increased impairment in frontal executive domains. Regarding neuropsychiatric disturbances, no specific behavior was more common in any of the PSP subtypes.

Conclusion

Motor deficits delineate the phenotypes included in currently accepted MDS-PSP criteria. Cognition and behavioural disturbances are common in PSP and allow us to distinguish this disorder from other neurological diseases, but they do not differentiate between PSP phenotypes.

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Data availability

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

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Acknowledgments

The authors thank all the patients and their families for their participation in this study.

Funding

In the last 12 months, Andrea Horta-Barba received a salary from the Research Institute of Sant Pau and received compensation for speaker activities from UCB and AbbVie. Javier Pagonabarraga has received honoraria as speaker or Member of Advisory Board for Zambon, Bial, UCB, Allergan, Abbvie, and Ipsen. Saül Martínez-Horta received a salary from the Research Institute of Sant Pau, has been awarded a grant from the Huntington’s Disease Society of America and received compensation for speaker activities from UCB, AbbVie and Roche. Laura Busteed is studying her Master of Science and has not received honoraria. Berta Pascual-Sedano received compensation for speaker activities from Abbvie, UCB and Ferrer. Ignacio Illán-Gala is supported by the Global Brain Health Institute (Atlantic Fellow for Equity in Brain Health). Juan Marín-Lahoz received a salary from the PERIS of the Generalitat of Catalonia. Ignacio Aracil-Bolaños has received a salary from the Research Institute of Sant Pau and research support from Carlos III Institute Research Grant. Jesús Pérez-Pérez is supported by a research grant from the Government of Spain (ISCIII). Frederic Sampedro is supported by a research grant from the Government of Spain (ISCIII). Helena Bejr-Kasem has received a salary from a pre-doctoral grant awarded by the Spanish Government (ISCIII), from the Research Institute of Sant Pau and received compensation for speaker activities in scientific meetings supported by Zambon, and non-financial support for congress attendance from Abbvie, Zambon and Allergan. Jaime Kulisevsky has received public research support from CIBERNED and Carlos III Institute, unrestrictive research support from Zambon and TEVA, and honoraria for lecturing and/or consulting from Zambon and TEVA.

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Authors and Affiliations

  1. Movement Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Mas Casanovas, 90-08041, Barcelona, Spain

    Andrea Horta-Barba, Javier Pagonabarraga, Saül Martínez-Horta, Laura Busteed, Berta Pascual-Sedano, Juan Marin-Lahoz, Ignacio Aracil-Bolaños, Jesús Pérez-Pérez, Frederic Sampedro, Helena Bejr-Kasem & Jaime Kulisevsky

  2. Universitat Autònoma de Barcelona (U.A.B.), Barcelona, Spain

    Andrea Horta-Barba, Javier Pagonabarraga, Saül Martínez-Horta, Berta Pascual-Sedano, Juan Marin-Lahoz, Ignacio Aracil-Bolaños, Jesús Pérez-Pérez, Helena Bejr-Kasem & Jaime Kulisevsky

  3. Institut d’Investigacions Biomèdiques- Sant Pau (IIB-Sant Pau), Barcelona, Spain

    Andrea Horta-Barba, Javier Pagonabarraga, Saül Martínez-Horta, Berta Pascual-Sedano, Ignacio Illán-Gala, Juan Marin-Lahoz, Ignacio Aracil-Bolaños, Jesús Pérez-Pérez, Frederic Sampedro, Helena Bejr-Kasem & Jaime Kulisevsky

  4. Centro de Investigación en Red-Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain

    Andrea Horta-Barba, Javier Pagonabarraga, Saül Martínez-Horta, Berta Pascual-Sedano, Ignacio Illán-Gala, Ignacio Aracil-Bolaños, Jesús Pérez-Pérez, Frederic Sampedro, Helena Bejr-Kasem & Jaime Kulisevsky

  5. Memory Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain

    Ignacio Illán-Gala

  6. Faculty of Health Sciences, Universitat Oberta de Catalunya (UOC), Barcelona, Spain

    Andrea Horta-Barba, Berta Pascual-Sedano, Helena Bejr-Kasem & Jaime Kulisevsky

Authors
  1. Andrea Horta-Barba
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  2. Javier Pagonabarraga
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  3. Saül Martínez-Horta
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  4. Laura Busteed
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  5. Berta Pascual-Sedano
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  6. Ignacio Illán-Gala
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  7. Juan Marin-Lahoz
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  8. Ignacio Aracil-Bolaños
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  9. Jesús Pérez-Pérez
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  10. Frederic Sampedro
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  11. Helena Bejr-Kasem
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  12. Jaime Kulisevsky
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Contributions

AH-B: research project conception, organization, execution, design. Formal analysis. Writing – original draft. JP: research project conception, organization, execution, design. Formal analysis. Writing – original draft. SM-H: research project conception, design. Formal analysis. Writing – review and critique. LB: research project organization and execution. Writing – review and critique. BP-S: research project organization and execution. Writing – review and critique. II-G: research project organization and execution. Writing – review and critique. JM-L: research project organization and execution. Writing – review and critique. IA-B: research project organization and execution. Writing – review and critique. JP-P: research project organization and execution. Writing – review and critique. FS: statistical analysis execution. Writing – review and critique. HB-K: research project organization and execution. Writing – review and critique. JK: research project conception, organization, design. Formal analysis. Writing – original draft.

Corresponding authors

Correspondence to Javier Pagonabarraga or Jaime Kulisevsky.

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Ethical standard

The study has been approved by the ethical committee of Sant Pau Hospital (Barcelona, Spain). Written informed consent was obtained from all participants and the study was conducted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Horta-Barba, A., Pagonabarraga, J., Martínez-Horta, S. et al. Cognitive and behavioral profile of progressive supranuclear palsy and its phenotypes. J Neurol 268, 3400–3408 (2021). https://doi.org/10.1007/s00415-021-10511-y

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